Short-term prognostic value of heart failure diagnosis in a contemporary cohort of patients with ACHD

Abstract Background Heart failure (HF) is one of the primary causes of premature death in patients with adult congenital heart disease (ACHD), yet population-specific data remains scarce. This study aims at assessing the short-term value of prognostic value of a heart failure diagnosis in patients with adult congenital heart disease, and its relation to ACHD classification systems such as the Bethesda disease complexity classification and the physiological severity classification. Methods This study included 3995 patients under active follow-up at our institution. We used a standardized definition of ACHD-HF. Predictors of the composite primary outcome: death, transplant or ventricular assist device (VAD) were identified using uni- and multivariable Cox proportional hazard models. Results Mean age was 35.7±13.3 years. According to the Bethesda disease complexity classification 33.5% of patients had simple defects, 55.6% moderate defects, and 10.9% severe defects. Based on the physiological severity classification, 18.0% of patients were in physiological stage A, 35.6% in physiological stage B, 42.2% in physiological stage C, and 4.2% in physiological stage D. The overall prevalence of ACHD-HF was 6.4%. During a median follow up of 1.8 (IQR 1.3–2.9) years, patients with ACHD-HF had a worse outcome with 27.3% reaching the composite primary endpoint of death, heart transplant or VAD implantation, compared to 1.4% of ACHD patients without HF. Event-free survival was 91.1%, 72.0% and 46.1% at 2, 4 and 6 years in patients with ACHD-HF, compared to 99.5%, 98.7% and 95.1% in ACHD patients without HF. In multivariable analysis the presence of heart failure (HR 4.6; 95% CI 2.9–7.2; p